Portopulmonary Hypertension Presence of pulmonary arterial hypertension in a patient with portal hypertension No other obvious causes (chronic lung disease, left heart disease, thromboembolism) 1) SCREEN - Transthoracic echocardiogram • Right ventricular systolic pressure (RVSP) - Estimates pulmonary artery pressure via tricuspid regurgitant velocity using Bernoulli principle. No consensus on definite cutoff - Previous studies suggested >50mmHg correlated with moderate (or worse) PPHTN • Peak tricuspid regurgitation velocity (TRV) - Cutoff > 2.8 m/s • Other signs: Right ventricular size, Right atrial pressure, Pulmonary artery diameter, Flattening of ventricular septum 2) DIAGNOSE - Right heart catheterization Needed for definitive diagnosis Diagnostic criteria: mPAP > 25mmHg, PVR > 3 Woods units, PA wedge pressure < 15mmHg TREATMENT: Endothelin receptor antagonists: - Mechanism: competitive antagonism of endothelin receptors, blocking the vasoconstricting effect of endothelin - Effect: improves functional class, exercise capacity, and cardiopulmonary hemodynamics Phosphodiesterase-5 inhibitors: - Mechanism: facilitates the vasodilatory effects of nitric oxide through reduced cGMP metabolism - Effect: improves functional class and cardiopulmonary hemodynamics Prostacyclin analogues: - Mechanism: causes vasodilation and reduced vascular smooth muscle proliferation - Effect: shown to improve cardiopulmonary hemodynamics Soluble guanylate cylase stimulators: - Mechanism: sensitizes soluble guanylate cyclase (sGC) to nitric oxide (NO) and directly stimualtes sGC receptors independent of NO - Effect: PATENT-1 study showed improved functional status and hemodynamics Liver transplantation: Consider in those who respond to medical therapy Lizzie Aby, MD @LizzieAbyMD #Portopulmonary #Hypertension #diagnosis #management #hepatology
